Importance of Clinical and Morphological Correlations in Diagnosing Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a clonal histiocytic disorder. The variable clinical manifestations from isolated bone lesion to multisystem disease can cause difficulties and delay in diagnosis. We report a 2 years and 8 months-old girl who presented with a 2 weeks history of persistent fever and weight loss associated with progressive abdominal distension. Physical examination revealed pallor, bilateral proptosis, seaborrheic dermatitis over the scalp and hepatosplenomegaly. Skull X-ray demonstrated multiple lytic lesions at the base and the skull vault. Bone marrow morphology showed numerous abnormal Langerhans cells (LCs) and foamy macrophages. The trephine immunohistochemistry (IHC) stains for CD1a, S-100 and CD68 were inconclusive. The diagnosis of multisystem Langerhans cell histiocytosis (MS-LCH) in this patient was based on the clinical presentation, radiological and morphological analysis. She subsequently received chemotherapy and currently she is on maintenance therapy with a good clinical response. LCH is a rare disease and although the IHC was inconclusive, the correlation of clinical, radiological and morphological data are essential for the diagnosis.

Detection of Partial G6PD Deficiency using OSMMR2000-D Kit with Hb Normalization

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide including Malaysia. Screening of cord blood for partial G6PD deficiency is important as they are also prone to develop acute haemolysis. In this study, we determined the prevalence of partial G6PD deficient in paediatric population aged 1 month-12 years and normal term female neonates using OSMMR-D kit with haemoglobin (Hb) normalization and compare it with florescence spot test (FST). A total of 236 children, aged between between 1 month-12 years and 614 normal term female neonates were recruited for this study. Determination of normal means for G6PD activity and; cut-off points for partial and severe deficiency were determined according to WHO Working Group (1989). Determination of prevalence for partial deficiency for both groups (female patient) was done using this enzyme assay kit and findings were compared with FST. In this study, 15.7% (18/115) female children were classified as partial G6PD deficient by quantitative enzyme method (G6PD activity: 4.23-5.26U/gHb). However, FST only detected 0.9% (1/115) with minimal G6PD activity. The prevalence of partial G6PD deficiency in female neonate group was 3.42% (21/614) by enzyme assay versus 0.49% (3/614) by FST. This study concluded that our routine screening method using FST was unable to diagnose female heterozygotes. We recommend using this quantitative enzyme assay method by OSMMR-D kit since it was more sensitive in detecting G6PD deficiency in female neonates compared to FST.

Foot care strategy for the newly diagnosed DM Type 2 patients with low educational and socio-economic background: a step towards future.

BACKGROUND AND AIMS: Diabetic foot ulcer is one of the major health problems that accounts for increased morbidity among the diabetic patients. Having good knowledge, good attitude and practice of managing the foot prevents the impending chronic co-morbidities of the disease. MATERIALS AND METHODS: This cross-sectional study was performed to assess the knowledge, attitude and practice on foot care among the newly diagnosed diabetic type 2 patients with low education and socio economic background. This study was conducted in one of the out patient clinics in a tertiary hospital. A set of questionnaire adopted from The Michigan Diabetes Research and Training Center (MDRTC), was used to assess 109 respondents based on their knowledge of diabetes mellitus, practice and attitude towards the condition and care of the feet. RESULTS: The overall finding on knowledge, practice and attitude had shown unsatisfactory result. There was no relationship between the knowledge, practice and attitude with care of the feet. Only 20 (18.3%) respondents had a high score on knowledge, 31 (28.4%) had practiced good habits and 5 (4.6%) showed positive attitude towards care of the feet. However, there was significant finding on the level of education and the knowledge of foot care (p=0.01); CONCLUSION: Strategies should be developed to overcome the longterm complications. As for the Muslim patients, ablution, the ritual practice of washing and cleaning both feet prior to the prayers may be a possible means of checking the feet for any diabetic foot complication.

The quality of life among coronary heart disease patients at a teaching hospital.

BACKGROUND AND AIMS: Quality of life after acute coronary heart disease amongst patients is important outcome factor in deliberations of patient's care. The main aim of the study was to examine the quality of life amongst acute CHD patients. MATERIALS AND METHODS: A cross sectional descriptive study was conducted after an acute attack amongst coronary heart disease (CHD) patients in Universiti Kebangsaan Malaysia Medical Centre (UKMMC).The Medical Outcomes Short Form 36 (SF-36) comprised of 36 items used to measure quality of life which comprised of 4 domains of physical component summary were physical function, role physical, bodily pain, and general health and 4 domains of mental component summary were vitality, social function, emotional role, and mental health. A total of 108 respondents were recruited for this study. RESULTS: The findings showed that CHD. Respondents possessed good level of quality of life with total score of (59 ± 22). The total score of physical domain had mean and SD of 56 ± 24, while the total scores of the mental domain had mean and SD of 62 ± 27. There were significant differences between the general health components of quality of life with educational status of the CHD patients with (F= 5.433, p<0.05). There were significant differences in role physical components of quality of life with income (F= 3.144, p<0.05). CONCLUSION: The cardiac rehabilitation program would have deliberately improved their needs and conditions whilst hospitalization. These results have implications in which CHD patients should be evaluated with regard to their continuity of care.

The ideal measurement of the quality of life in post stroke patients: an urban study.

OBJECTIVE: There is paucity of literature on the measurement of the quality of life in post stroke patients in the developing countries. The main objective of this study was to determine the quality of life (QOL) of post stroke patients. MATERIALS AND METHODS: A cross-sectional study was conducted on 107 post stroke patients in two centers. The data was obtained during the period of January 2009 till May 2009 using purposive sampling method. A total of 68 males and 39 females participated in this study. Stroke Specific Quality Of Life (SS-QOL) version 2.0 was used. Cronbach's alpha values for SS-QOL ranged between 0.73 and 0.89. RESULTS: The mean ± SD of the QOL score was 141.79 ± 40.32. The mean ± SD of the age was 65.47 ± 11.79 years. Result showed significant negative association between age and QOL of post stroke patients (r = - 0.199, p= 0.040). Domains mostly affected by stroke compared to pre-stroke were mobility (69%) and energy (64%). There were two domains in SS-QOL significantly predicted the QOL of post stroke patients. These domains were work and productivity (ß= 2.277, t= 2.145, p= 0.035) and thinking (ß= 1.927, t= 2.567, p= 0.012). CONCLUSION: Few items from this measurement tool may not be appropriate in a developing country because of the different cultural background. There is a need to develop appropriate post stroke patient measurement tool based on local practice.

Perceived Stress Related to Nursing Education and Its Influence on Nursing Students’ Academic and Clinical Performance

Stress has a negative effect on student nurses well-being and can impede learning or motivate them and is conducive to learning. This study examined the perceived stress and factors that influenced daily students’ life among both the Diploma and Bachelor of Nursing students. A total of 241 nursing students were involved in this research project. Findings of this study indicated that junior nursing students (<21 years) of both cohorts perceived significantly more stress than older (≥21 years) nursing students. Second year students of the Diploma group claimed being more stressed than their peers (1st and 3rd years). Personal problems were the main issue of concern among the second year Diploma students. Workload of nursing education and fear of failing the nursing programme were the major stressors among the first year nursing students of the Bachelor cohort as compared to their seniors. A mixture of negatively and positively significant correlations was observed between academic work and clinical scores among the Diploma and Bachelor of Nursing students respectively. Both the Diploma and Bachelor of Nursing students’ stress did not influence their GPA scores. Negatively significant correlation was noted between stress and clinical scores among the Diploma of Nursing students. A negatively significant correlation was found between age and CGPA scores among the Bachelor cohort. Both male and female Diploma and Bachelor nursing students perceived no differences in stress related to their academic work. The findings of this study provide important information for nursing education at the institute examined in this study, and have implications for future research.

Workplace Violence Experienced by Nurses in Universiti Kebangsaan Malaysia Medical Centre

Lately, increasing workplace violence is a problem that requires serious attention by the management of the healthcare sector. This study was carried out to examine workplace violence experienced by nurses in the Universiti Kebangsaan Malaysia Medical Centre. It aimed to identify the category of nursing staff who formed the highest number of victims, the perpetrators and the common types of workplace violence. This was a descriptive cross-sectional study carried out over a three month period on 55 nurses who had participated voluntarily in this study. The demographic data and data on the violent incident were collected by using a 21-item questionnaire. The reliability and validity with Cronbach’s alpha was 0.872. Information on the most common types of violence, victims and the perpetrators was also collected. The study revealed that workplace violence among the nursing staff was 3.7% with an average of 1.2% being abused per month and one nurse being abused every other day. The study also revealed that the staff nurses were the most common victims and the perpetrators were mainly the patients (40.6%) and the patients’ relatives (37.5%). The most common forms of violence were verbal abuse (31.9%) and verbal threat (23.7%). Physical violence was experienced by 44.4% of the victims. These results suggest that workplace violence is a serious problem which should be addressed in order to create a safe environment for the nurses.

Expression of multidrug resistance (MDR) proteins and in vitro drug resistance in acute leukemias.

The expression of the multidrug resistance (MDR) proteins may influence the outcome of treatment in patients with acute leukemia. The aim of this study was to determine the IC50 of cytotoxic drugs (cytosine arabinoside, ara-C and daunorubicin, dnr) using the in vitro 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)2H-tetrazolium, inner salt (MTS) assay method. A total of 82 newly diagnosed acute leukemia cases (43 adult myeloid leukaemia, AML cases and 39 acute lymphoblastic leukaemia, ALL cases) and 16 relapsed cases (8 AML cases and 8 ALL cases) were studied. The MTS assay was performed using two cytotoxic drugs, dnr and ara-C. Cells were incubated with different concentrations of drugs for 4 days and the IC50 was extrapolated from the viability curve. In newly diagnosed cases, we found that childhood ALL samples showed higher IC50 values of dnr (0.040 +/- 2.320) compared to adult AML samples (0.021 +/- 0.158). In contrast, newly diagnosed adult AML samples showed higher IC50 values of ara-C (0.157 +/- 0.529) compared to childhood ALL samples (0.100 +/- 2.350). In relapsed cases, two samples of childhood ALL showed IC50 values of dnr (0.910 +/- 1.760) and ara-C (1.310 +/- 2.390), which was higher compared to childhood AML samples (0.129 +/- 0.214 and 0.210 +/- 0.003, respectively). However, there was no correlation between IC50 values of these drugs tested with clinical outcome. In conclusion, we found that MTS assay is an easy, rapid and non laborious method to study in vitro drug resistance in acute leukaemia cases.

Sharps Injury in Hospital Universiti Kebangsaan Malaysia (HUKM): Experiences of Health Care Workers and Students

Sharps injury among health care workers in HUKM has received serious attention lately due to the possible transmission of diseases like HIV, Hepatits B and Hepatitis C, which can cause serious consequences for the victim. The aim of the study was to gain a deeper understanding of the emotional problems experienced by health care workers who had incurred a sharps injury and the coping strategies adopted by them to overcome the problem. A 28 item semi-structured questionnaire was designed by the supervisors and the researcher as there was no existing questionnaire to be adopted, as no research had been carried out in this area prior to this. The target group consisted of 64 health care workers of different categories working in HUKM. Data was collected over a period of five months from November 2000 to March 2001. The results of the study revealed that the most frequently injured were the staff nurses, nearly 90% of whom were emotionally upset and were worried that they might contract a disease. There was no significant difference in the emotional stress experienced by the different categories of the health care workers, all were equally upset about the injury but each category of staff adopted different methods to overcome the tragedy. Open comments and opinions about prevention and the management of the sharps injury in HUKM as given by the respondents here are taken into account. Several views given by the participants were explored and discussed. Finally, several implications for nursing and recommendations have been presented.

A study on anxiety and depression level among high risk inpatient pregnant women in an obstetric ward

Pregnancy is perceived by many pregnant mothers as a period of happiness in anticipation of motherhood. Not all pregnant mothers experience cheerfulness as some may experience a high anxiety and depression level for unknown reasons. The purpose of this study was to determine the level of anxiety and depression among high risk pregnant women and the factors that contribute to their level of anxiety and depression. A descriptive cross-sectional study was conducted on 38 high risk mothers whose stay in hospital exceeded more than three days in the obstetric ward of Hospital Universiti Kebangsaan Malaysia. A self assessment questionaire "Hospital Anxiety Depression Scale"was used to measure the level of anxiety and depression among high risk pregnant women. Of the 38 participants, 16 (42.1%) women experienced a mild level of anxiety and 22 (57.9%) experienced a severe level of anxiety. Seventeen (44.7%) women was classified as having mild depression and 21 (55.3%) severe depression. The factors contributing to the level of anxiety and depression, include those related with "lack of information on disease", "family matters" and "finance". High risk pregnant women in this study experienced a significant level of anxiety and depression during their stay in hospital. It is therefore important for nurses and doctors to be aware and sensitive to the influencing factors that cause anxiety and depression as to enable high risk pregnant mothers to enjoy their pregnancy and childbirth.

G6PD deficiency with hemolytic anemia due to a rare gene deletion--a report of the first case in Malaysia.

A 2-year-old Chinese boy was referred to Hospital UKM for investigation of recurrent episodes of dark-coloured urine and pallor since birth. He was born prematurely at 34 weeks gestation and developed severe early-onset neonatal jaundice requiring exchange blood transfusion. Screening at birth showed Glucose-6-phosphate dehydrogenase (G6PD) deficiency. On admission, physical examination revealed pallor, jaundice and mild hepatomegaly. Results of laboratory investigations showed a hemoglobin level of 11.0 g/dl with a hemolytic blood picture, reticulocytosis of 20% and red cell G6PD activity reported as undetectable. The patient's DNA was analysed for G6PD mutations by PCR-based techniques and DNA sequencing and results showed a 24 bp deletion of nucleotide 953-976 in the exon 9 of the G6PD gene. DNA analysis was also performed on blood samples of the patient's mother and female sibling confirming their heterozygous status, although both showed normal red cell G6PD activity levels. The patient was discharged well and his parents were appropriately advised on the condition and the importance of taking folic acid regularly. This is a first case report in Malaysia of G6PD deficiency causing chronic-hemolytic anemia. The rare 24 bp deletion causes the G6PD Nara variant, previously reported only in two other unrelated males, a Japanese and a Portuguese both with chronic hemolytic anemia.

Detection of immunoglobulin gene rearrangement in B-cell malignancies.

Rearrangement of the immunoglobulin heavy chain (IgH) gene has been used as a marker of lineage and clonality in the diagnosis of B lymphoproliferative disorders. A number of PCR-based techniques have been developed to overcome the disadvantages of Southern blotting, the standard technique in detecting IgH gene rearrangement. Using an established seminested PCR technique with consensus primers to the V and J regions of the IgH gene, we analysed DNA prepared from peripheral blood and/or bone marrow specimens from 30 cases of known B cell malignancies (16 chronic lymphocytic leukemia, 11 acute lymphoblastic leukemia and 3 Non-Hodgkin Lymphoma), 3 cases of T lymphoproliferative disease and 3 cases of reactive lymphocytosis diagnosed in Hospital UKM to detect rearranged IgH gene. We found that monoclonality as represented by the presence of rearranged IgH gene were demonstrated in all the 30 cases. The PCR findings showed 100% concordance with the Southern blot analysis results which also showed rearranged IgH bands in all the 30 cases. We also found that none of the cases of T lymphoproliferative diseases and reactive lymphocytosis showed presence of rearranged IgH band, suggesting that the amplification using the IgH primers is lineage-specific. In conclusion, we find the PCR a useful method to detect IgH gene rearrangement in peripheral blood and bone marrow specimen. Since the PCR results are comparable to that of the Southern blotting in demonstrating B cell monoclonality and owing to its many advantages we feel that it can replace the Southern blot technique for the diagnosis of B cell malignancies.

Evaluating in-house anti-serum against B cells with flow cytometry.

The applications of antibodies, be it monoclonal or polyclonal, in the diagnostic and research fields are well established. The disadvantage is the high cost of commercially available antibodies. In a diagnostic establishment like ours which also functions as a training ground for laboratory related personnel, it is beneficial to be able to produce in-house reagents. Therefore, we have undertaken this project to produce a rabbit polyclonal antibody against B lymphocytes. We found that the rabbit was a good choice because the titre of antibody produced was high and positive reactions were still detected at a dilution of 1:38400. The antibody showed significant positive reaction only with the lymphocyte subpopulation. A positive reaction was observed between the immunized rabbit serum and B lymphocytes but not T lymphocytes. This shows that the antibody was B lymphocyte specific. There was a positive correlation between the percentage of B lymphocytes labelled using the commercial anti-CD19 monoclonal antibody and the in-house polyclonal antibody (n = 13, r = 0.7, p = 0.02). However, the percentage of cells labelled by the in-house polyclonal anti-B was lower than that by the commercial monoclonal anti-CD19. The fluorescence intensity of the polyclonal antibody was lower than that of the monoclonal. In general, the performance of the in-house polyclonal antibody can be considered as satisfactory. The rabbit serum was stored at -20 degrees C and no significant loss of activity was detected for over a period of 19 months.

In vivo evidence of all-trans retinoic acid inducing maturation in acute promyelocytic leukaemia.

All-trans retinoic acid therapy induces maturation in acute promyelocytic leukaemia. We document in vivo evidence of differentiation by all-trans retinoic acid in a case of acute promyelocytic leukaemia which was characterized by cytoplasmic vacuolations.